[Brief
History]
The 63 year-old male was a case of
general health before and denied any systemic disease. This
time, the patient suffered from petechiae over four limbs,
easy bruising, dizziness, easy fatigue and exertion dyspnea in
recent 2~3 months. No tarry stool or bloody stool, no
orthopnea, no fever, no chills, no cough, no sputum was noted.
There was also no family history of bleeding tendency. Due to
the symptoms and signs described above, the patient visited
medical help and initial CBC revealed anemia and
thrombocytopenia (WBC 3510/ul, Hb 7.2 g/dl, PLT 120000/ul) and
then transferred to our hospital for further evaluation
treatment.
[Laboratory
Date]
After admitted to our ward, a serial survey were performed
a listed as below:
Peripheral blood
smear: Thrombocytopenia and Rouleaux formation
was noted. Biochemistry: TP/Alb: 9.48/3.93
g/dl, A/G ratio: 0.7 Beta-2 Microglobulin : 598 ug/dl, LDH 517 u/L
BUN/Cr: 25/1.6 mg/dl
Immunoglobulin:IgG/A/M:
6880/11.5/9.54 mg/dl Electrolyte: Na/K/Cl: 141/4.1/103
mEq/L, Total calcium: 10.3 mg/dl Protein Electrophoresis: monoclonal
gammopathy. Immunoelectrophoresis: IgG l
monoclonal gammopathy, Bence-Jones
proteinuria. BMA: Increased cellularity (70%), plasma cell
increased (80%) with bizarre formation. Abdominal echo:
splenomegaly. Hepatitis
marker: HBsAg (-), Anti-HCV
(-)
[Treatment and
Course]
According to the above
survey, the patient was diagnosed as multiple myeloma (IgG,
ltype).
Besides, the stage IIIA was also diagnosed because of
hemoglobin level < 8.5 g/dl and serum creatinine level <
2 mg/dl. Due to advance stage, the patient received systemic
chemotherapy with melphalan (8 mg/m2) and prednisolone (25~60
mg/m2) administered for 7 days every 4~6 weeks. After several
courses of treatment, the IgG level may reduce to around 2
mg/dl.
[Discussion]
多發性骨髓瘤是一種plasma
cells惡性增生的疾病,其確切發病原因仍未確定。多發性骨髓瘤的發生率隨著年紀的增加而增加,診斷的median age是68
歲,很少有患者在40歲之前被診斷出來。
骨頭疼痛是多發性骨髓瘤最常見的症狀,影響大約70%的患者。這種疼痛常會因為活動而加重。其原因通常是因為proliferation
of tumor cells以及activation of osteoclasts that destroy the
bone所造成。骨頭病灶大多是蝕骨性的 (osteolytic),少有new bone
formation的情形。第二常見的問題是容易遭受細菌感染。多發性骨髓瘤患者最常發生的感染是pneumonia以及pyelonephritis。在肺炎最常見的菌種包括:Streptococcus
pneumoniae、Staphylococcus aureus、Klebsiella
pneumoniae,在泌尿道最常見的菌種則包括:Escherichia coli以及其他gram-negative
organisms。大約有25%的患者是以反覆性感染來表現的。此外,有25%的患者會有腎臟衰竭的情形。造成腎臟衰竭的原因包括:高血鈣、Amyloid沈積在glomerulus、泌尿道反覆感染、或是腎臟被myeloma
cells所浸潤。其他的臨床表現包括:貧血、高血鈣、黏滯係數過高…等。
診斷方面,多發性骨髓瘤的triad包括:(1) marrow plasmacytosis
> 10%,(2) lytic bone lesions, (3) Serum and/or urine M
component。但即使患者沒有osteolytic bone lesion,若是患者有marrow
plasmacytosis以及逐漸上升的M component仍然可以診斷為多發性骨髓瘤。但是,測不到M
component的多發性骨髓瘤患者則是不到1%。Staging System主要根據hemoglobin、serum
calcium、lytic bone lesion、M-component production而分為Stage I,
II, III;並根據serum creatinine level再細分為A or
B。b2-microglobulin是單一變項中與預後關係最密切的。
大多數的多發性骨髓瘤患者是必須受治療的,包括所有的stage II、stage III、以及部分的stage
I患者必須接受systemic combination
chemotherapy;治療可以改善survival以及生活品質。傳統standard
treatment是間歇性的給予一種Alkylating
agent加上prednisolone,每次給予4~7天,每4~6星期給予一次。在Alkylating
agent中最常用的Melphalan,其常用劑量為8
mg/m2/day;至於prednisolone的常用劑量為25~60
mg/m2/day。至於治療最適當的duration目前仍未確定,大部分的醫師會每4~6
weeks一個療程持續1~2年。停藥之後疾病常常會再復發,而且大都在一年之內復發。再次給予治療,2nd response
rate仍然可以達到80%。其他的治療方式有自體或是異體造血幹細胞移植,thalidomide等。預後方面,有15%的患者
在診斷後的三個月內會死亡,其後每年增加15%的死亡率。病患在發生acute terminal phase
(通常以pancytopenia表現且對治療沒有反應)之前,通常會有2~5年的chronic course。
|